The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

PurposeWe developed the congenital diaphragmatic hernia congenital prognostic index (CDH-CPI) to incorporate all known prognostic variables into a single composite index to improve prognostic accuracy. The purpose of this study is to examine the ability of the CDH-CPI to predict survival in patients with left-sided congenital diaphragmatic hernia and to determine if the index has a stronger correlation with survival than each of the individual components.MethodsA retrospective review of patients with left-sided congenital diaphragmatic hernia between 2004 and 2010 was conducted. Ten prenatal parameters of the CDH-CPI were collected, total score was tabulated, and patients stratified according to total score and survival.ResultsSixty-four patients with a prenatal diagnosis of left-sided congenital diaphragmatic hernia were identified. Patients with a CDH-CPI score of 8 or higher had a significantly higher survival than patients with a CDH-CPI score of lower than 8. The CDH-CPI has the strongest correlation with survival compared with the individual parameters measured. The CDH-CPI correlates with extracorporeal membrane oxygenation use, and 75% of patients with a score of 5 or lower were placed on extracorporeal membrane oxygenation.ConclusionsThe CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used.     

Long-term follow-up of patients with high-risk congenital diaphragmatic hernia

Background/Purpose

Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established.

Methods

Thirty-three children with ages 4.1 ± 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy.

Results

No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung.

Conclusion

These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.     

High altitude simulation testing in patients with congenital diaphragmatic hernia

Aim of studyCongenital Diaphragmatic Hernia (CDH) is associated with lung hypoplasia and pulmonary hypertension. Many patients receive care in specialty centers requiring air travel upon discharge and for subsequent follow-up care. Premature infants can experience significant hypoxia in flight, but this has not been studied in the CDH population. This report describes our center's experience with simulated altitude testing among CDH patients.MethodsIn a single center retrospective cohort study, CDH patients who underwent a High Altitude Simulation Test (HAST) from 2006 to 2019 were analyzed. HAST simulates increased altitude by reducing oxygen tension to an FIO2 of 0.15. Patients were tested only when flight was anticipated. Patients requiring oxygen were challenged on their baseline requirement. To pass, patients had to maintain oxygen saturation > 90%, and 94% if diagnosed with pulmonary hypertension. Supplemental oxygen was titrated as needed to achieve this goal.ResultsOf twenty patients tested, only six (30%) passed on their first attempt. Ten (50%) eventually passed, after an average of 3.2 additional attempts over 1.8 years. No patient passed initially who utilized ECMO support, diaphragmatic agenesis, or had elevated right ventricular pressure on echocardiogram. All patients achieved the targeted SpO2 with supplemental oxygen.ConclusionCDH patients experience hypoxia when exposed to the simulated hypobaric nature of air travel and therefore may become hypoxic in flight, requiring oxygen supplementation. Disease severity seems to correlate with risk of in-flight hypoxia. This data suggests that CDH patients should be screened to assess their need for supplemental oxygen to ensure safe air travel.Level of evidenceLevel 4 case series.     

Late-presenting congenital diaphragmatic hernia

Background

Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database.

Methods

Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively.

Results

Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival.

Conclusions

Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.     

Bilateral congenital diaphragmatic hernia

Background/Purpose: CDH occurs in approximately 1 in 2,450 live births. Bilateral CDH, previously identified through a limited number of case reports, is extremely rare. The care of CDH patients is a challenge for neonatologists and surgeons. This report details the management and outcome of patients with bilateral CDH. Methods: Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with bilateral CDH were reviewed retrospectively. Data were analyzed using the [Chi ]² test. Results: A total of 1833 patients were entered in the database, 17 of these had bilateral CDH (0.9%). Eleven were boys. The average birth weight was 2.6 kg. The average gestational age was 36.8 weeks. Sixteen patients experienced early distress requiring intubation (12 immediately), and 4 were placed on extracorporeal membrane oxygenation (ECMO). Seven patients were diagnosed prenatally. Twelve patients (70%) were found to have other anomalies, 3 had chromosomal abnormalities and 7 had cardiac anomalies. These included tetralogy of Fallot (TOF), VSD, absence of the pericardium, coarctation of the aorta (2), accessory SVC with aortic coarctation, and ASD with TOF. Only 9 of 17 (53%) patients underwent surgical repair (6 primary, 3 patch). Mortality rate was 65% compared with 33% of patients with unilateral CDH (P = 0.01). Seven patients died within 48 hours of birth. There was no significant difference in survival based on gender, weight, gestational age, presence of anomalies, or prenatal diagnosis. Conclusions: The management of infants with bilateral congenital diaphragmatic hernia remains a difficult problem with a significant mortality. Bilateral congenital diaphragmatic hernia is associated more frequently with other major anomalies than unilateral congenital diaphragmatic hernia and should prompt an evaluation for further anomalies. J Pediatr Surg 38:522-524.     

Fetal anesthesia in cesarean section for patients with congenital diaphragmatic hernia

We experienced two cases of anesthesia for cesarean section in patients with prenatally diagnosed congenital diaphragmatic hernia (CDH). Fentanyl/midazolam anesthesia was selected for fetal sedation at birth. The blood samples were obtained from the maternal vein, umbilical vein and umbilical artery for measuring blood drug concentrations during anesthesia. Midazolam concentrations in umbilical vein were 137 and 256 ng.ml-1. Spontaneous breathing and movement of the newborns were restrained in both cases and these sedations facilitated preoperative general management for newborns with CDH.     

Predictors of early lung function in patients with congenital diaphragmatic hernia

Purpose

Long-term pulmonary outcomes of congenital diaphragmatic hernia (CDH) have demonstrated airflow obstruction in later childhood. We examined pulmonary function data to assess what factors predict lung function in the first three years of life in children with CDH.

Methods

This was a retrospective study of patients treated for CDH who underwent infant pulmonary function testing (IPFT) between 2006 and 2012. IPFT was performed using the raised volume rapid thoracoabdominal compression technique and plethysmography.

Results

Twenty-nine neonates with CDH had IPFTs in the first 3 years of life. Their mean predicted survival using the CDH Study Group equation was 63% ± 4%. Fourteen infants (48%) required extracorporeal membrane oxygenation (ECMO). The mean age at IPFT was 85.1 ± 5 weeks. Airflow obstruction was the most common abnormality, seen in 14 subjects. 12 subjects had air trapping, and 9 demonstrated restrictive disease. ECMO (p = 0.002), days on the ventilator (p = 0.028), and days on oxygen (p = 0.023) were associated with restrictive lung disease.

Conclusion

Despite following a group of patients with severe CDH, lung function revealed mild deficits in the first three years of life. Clinical markers of increased severity (ECMO, ventilator days, and prolonged oxygen use) are correlated with reduced lung function.     

17例先天性膈疝的外科治疗

目的总结１７例先天性膈疝的外科治疗经验,以提高治愈率。方法回顾性分析１７例先天性膈疝的诊断与治疗资料。结果全组１７例患儿中,１４例（占８２．４％）痊愈,３例（占１７．６％）死亡。结论正确诊断、早期急诊手术和术后精细护理是手术成功的关键。     

Fryns syndrome in children with congenital diaphragmatic hernia

Purpose: Fryns syndrome is characterized by multiple congenital anomalies including Congenital Diaphragmatic Hernia (CDH), and has a reported poor prognosis with a survival rate during the neonatal period of approximately 15%. This report details the management and outcome of patients with Fryns syndrome and CDH. Methods: Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with Fryns syndrome were reviewed retrospectively. Results: A total of 1,833 patients were entered in the database, 23 of these had Fryns (1.3%). All patients experienced early distress requiring intubation. Ten patients (43%) were found to have other major anomalies. Seven patients underwent surgical repair at an average age of 7.5 days (range, 6 hours to 14 days). Mortality rate was 83% compared with 33% of patients with unilateral CDH (P = .01). Ten patients died within the first 24 hours. The parents of 6 patients withdrew support. Of the 4 survivors, 3 have marked developmental delay, whereas the fourth has not yet undergone formal assessment. Conclusions: The prognosis of infants with Fryns syndrome and congenital diaphragmatic hernia remains grim. Early genetic counseling and recognition of lethal anomalies may assist in determining which patients may survive.     

Mortality prediction in congenital diaphragmatic hernia

Background

A validated risk stratification tool for congenital diaphragmatic hernia (CDH) is required for accurate outcomes analyses. Existing mortality-predictive models include those of the CDH Study Group (CDHSG) based on birth weight and 5-minute Apgar score, the Canadian Neonatal Network (CNN) based on gestational age and admission score in Score for Neonatal Acute Physiology version II, and the Wilford Hall/Santa Rosa clinical prediction formula (WHSR_PF) derived from blood gas measurements. The purpose of this study was to evaluate the calibration and discrimination of these predictive models using the Canadian Pediatric Surgical Network dataset.

Methods

Neonatal risk variables and birth hospital survivorship were collected prospectively in 11 perinatal centers, between May 2005 and October 2006. Actual vs predicted outcomes were analyzed for each equation to measure the calibration and discrimination of each model.

Results

Twenty (21.2%) of 94 infants with CDH died during birth hospitalization. The CDHSG model demonstrated superior discrimination (area under the receiver operator characteristic curve = 0.85; CNN = 0.79; WHSR_PF = 0.63). Model calibration reflected by the Hosmer-Lemeshow P value was poorest with the WHSR_PF = 0.37 and comparable between CDHSG and CNN (0.48 and 0.46, respectively).

Conclusion

Predictive outcome models are essential for risk-adjusted outcome analysis of CDH. The ideal predictive equation should prove robust across CDH datasets.     

Anesthesia for congenital diaphragmatic hernia

Hypoplasia of the lungs is the cause of the high mortality of newborns with diaphragmatic hernia. Survival depends mainly on the development of the contralateral lung. Eighty percent of diaphragmatic hernias are postolateral hernias of the left side. The most serious postoperative complication is a relapse into fetal circulation with increased pulmonary vascular resistance and right-to-left shunting (Fig. 2). The clinical signs of diaphragmatic hernia are cyanosis and tachypnea. Intermittent suction via a nasogastric tube and early intubation without mask ventilation should be performed. The inspiratory pressure should not exceed 25 cm H2O to minimize the risk of pneumothorax. Survival of the baby is unlikely if the initial blood gas analysis shows pH less than 7.10, pO2 less than 50 mmHg, and pCO2 greater than 65 mmHg. Hypothermia should be strictly avoided because it leads to increased oxygen consumption. Intraoperative monitoring should include a precordial stethoscope, ECG, blood pressure, and rectal temperature. Anesthesia is maintained with fentanyl 0.02-0.03 mg/kg body wt. and pancuronium 0.08-0.1 mg/kg. One dose of atropine (0.02 mg/kg) is administered before fentanyl. Intraoperative ventilation is performed by hand or by use of a Siemens Servo ventilator. Thirty newborns were anesthetized for repair of a congenital diaphragmatic hernia with no intraoperative complication and an overall mortality of 27%.     

Bilateral congenital posterolateral diaphragmatic hernia

The 11th recorded case of bilateral congenital posterolateral diaphragmatic hernia is here presented. Left-sided diaphragmatic hernia was corrected surgically, while the baby expired because of respiratory failure on the 31st operated day. Autopsy revealed that the baby had right posterolateral diaphragmatic defect with sac. From this experience, it might be said that at surgery for left-sided hernia, right diaphragm should be examined carefully.     

Intrapericardial diaphragmatic hernia: a rare type of congenital diaphragmatic hernia

An extremely rare case of congenital intrapericardial diaphragmatic hernia is presented. Since 1981, only 14 cases have been reported in the literature. A 5-year-old girl presented with dyspnea on exertion and easy fatigability. Computed tomography was suggestive of an anterior diaphragmatic hernia. Laparoscopy followed by successful open repair of hernia was performed.     

Effectiveness of diaphragmatic repair using an abdominal muscle flap in patients with recurrent congenital diaphragmatic hernia

Background/Purpose

Recurrent congenital diaphragmatic hernia (Re-CDH) is a well-known complication of CDH repair. Although surgical treatment is required for Re-CDH, the question regarding which technique is the optimal treatment has not yet been elucidated. We performed a repair using an abdominal muscle flap (AMF) for Re-CDH and herein report our experience.

Patients and Results

Four patients using AMF had left Re-CDH. The patient's age at operation was around 1 year old in all patients. In the first repair during the neonatal period, a Gore-Tex patch was required for the large defect in 3 patients, whereas a primary closure was selected in 1 patient because the anterior diaphragm remained as a partial remnant. In the 3 patients in whom a Gore-Tex patch was used in the first operation, recurrence occurred twice. All of the patients suffered from both pulmonary hypertension and growth retardation. A repair using AMF (the flap of both the internal oblique and the transversalis abdominal muscle) was performed in all patients. The clinical course was good in all patients, with an improvement of both pulmonary hypertension and growth retardation without any recurrence of CDH.

Conclusion

The use of an AMF was thus found to be an effective technique for repairing a large defect in Re-CDH.